Langerhans cell histiocytosis

Zoran Rumboldt, Andrea Rossi

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Specific Imaging Findings. Cranial MRI findings in Langerhans cell histiocytosis (LCH) include: (1) lesions of the facial bones, skull base, and calvarium; (2) intracranial extra-axial lesions (hypothalamic–pituitary region, meninges, circumventricular organs); (3) intra-axial changes; and (4) cerebral atrophy. Intracranial findings are typically associated with central diabetes insipidus (DI) and characterized by lack of high signal intensity of the posterior pituitary gland on T1-weighted images (from loss of ADH storage granules) and thickened enhancing pituitary stalk, which measures over 3 and even 7 mm in diameter. However, the posterior pituitary bright spot may persist in DI patients; on the other hand, other disease processes may present in a similar fashion. Hypothalamic (along the floor of the third ventricle) involvement with contrast enhancement can be seen, while pituitary and optic chiasm infiltration are found in some cases. In a minority of patients the infundibulum may be very thin and thread-like, under 1 mm in diameter; it can even be normal on MRI. The pineal gland may also show mild enlargement and contrast enhancement. Progressive reduction in size of the anterior pituitary on MRI is associated with a higher risk of additional endocrine defects. Repeated MRI studies in DI patients are of limited value for assessing a response to therapy, but are important for monitoring bone lesions and possible parenchymal CNS disease. Pertinent Clinical Information. LCH mainly affects children between 2 and 5 years of age with widely varying clinical manifestations and it may also occur in adults. CNS is affected in around 16% of patients, and DI is the most common manifestation (in 25%), followed by GH deficiency (in 10%) – usually diagnosed years after DI. However, a number of other diseases can cause DI and 30–50% of cases are considered idiopathic. LCH is usually self-limited in the absence of organ dysfunction. Brain involvement appears to be the single most important factor in determining quality of life.

Original languageEnglish
Title of host publicationBrain Imaging with MRI and CT: An Image Pattern Approach
PublisherCambridge University Press
Pages89-90
Number of pages2
ISBN (Print)9781139030854, 9780521119443
DOIs
Publication statusPublished - Jan 1 2010

ASJC Scopus subject areas

  • Medicine(all)

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